Diagnostic and surgical challenges of a giant pheochromocytoma in a resource limited setting—A case report

Liu

et al. 2020

Preprint

Introduction: Pheochromocytoma (PCC) is a rare tumor which derives from adrenal medulla, when maximum diameter of pheochromocytoma is greater than 10CM, it is divided into Giant pheochromocytoma(GPCC), which is extremely rare and usually asymptomatic.Clinically, a huge and asymptomatic adrenal pheochromocytoma was usually misdiagnosed as other types of tumors, which result in notable increase of complication rates and death rates.Case presentation:In this case report, we described a clinically asymptomatic GPCC patient. Diagnosis:According to computed tomography (CT) scan, nuclear magnetic resonance(MR) scan, the patient is initially diagnosed as liposarcoma.After laparotomy, biochemical detection of catecholamine (CA) intermediate metabolites methoxyepinephrine (MN) and methoxy norepinephrine (NMN) and the pathological examination , diagnosis of GPCC was obtained. Intervention：The laparotomy was suspended and a diagnosis of pheochromocytoma was confirmed, because of unstable blood pressure and hypertension during separating and moving the mass during surgery. After consultation of multi-disciplinary team, adequate preoperative preparation was conducted according to the procedure of PCC surgical preparation. By the end of the regular phenoxybenzamine and intravenous fluids treatment for three weeks, the blood pressure of the patient was kept at an acceptable average. Therefore, the patient underwent the operationagain for radical resection. Outcome: After the operation, CA was basically normal in rechecking process, and the tumor was successfully removed. Pathological diagnosis of the mass after operation: Immunohistochemical resulted conformed to (epigastrium) pheochromocytoma. Immunohistochemistry: CgA(+), Inhibin-α(-), ki-67(<1%+), Syn(+). Pheochromocytoma had the definite pathological diagnosis. Conclusion: GPCC has the high diagnostic identification difficulty, it should be combined with imaging examination and biochemical measurement to identify. Before operation, the detailed imageological examination provides important reference for excision and surgical planning. Individualized and multi-disciplinary cooperation of management strategy in perioperative period should be recommended. Patients should conduct long-term follow-up.

Section: Discussionmentioning

confidence: 74%

Case report: A Re-operation Case of Asymptomatic Left Giant Pheochromocytoma and Literature Review

Liu

et al. 2020

Preprint

“…In total, 50 published studies were identified by the PubMed search engine when employing the term “PASS pheochromocytoma”. After an initial assessment of eligibility, including the presence of a PASS stratified pheochromocytoma cohort as well as an exclusion of irrelevant studies and reviews, 28 original studies and case reports were initially included in the review [1,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29]. After careful revision of each study, eight additional studies were disqualified from inclusion based on the failure to meet our fourth inclusion criterion of “clearly identified subgroup information (number of tumor samples with and/or without evidence of synchronous or metachronous evidence of malignancy respectively, with the associated PASS score clearly identifiable)” [5,6,8,9,13,14,18,28].…”

Section: Resultsmentioning

confidence: 99%

“…After careful revision of each study, eight additional studies were disqualified from inclusion based on the failure to meet our fourth inclusion criterion of “clearly identified subgroup information (number of tumor samples with and/or without evidence of synchronous or metachronous evidence of malignancy respectively, with the associated PASS score clearly identifiable)” [5,6,8,9,13,14,18,28]. Therefore, a total of 20 studies were included in the meta-analysis [1,3,4,7,10,11,12,15,16,17,19,20,21,22,23,24,25,26,27,29]. These studies and the associated outcomes are detailed in Table 1 and illustrated in Figure 2A.…”

Section: Resultsmentioning

confidence: 99%

“…When applying a cut-off score of ≥4, the PASS algorithm correctly identified all 33 metastatic PCCs as no case scored <4—thereby ensuring a sensitivity of 100% and a risk stratification model with the potential to correctly “rule in” metastatic PCCs [1]. The PASS algorithm has since been assessed in several studies [3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29], of which numerous has verified the value of this approach and some which could not wholly reproduce the original findings. However, the algorithm also carries inter- and intraobserver variation, and the clinical value of the method has been debated [8,10].…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

The Value of Histological Algorithms to Predict the Malignancy Potential of Pheochromocytomas and Abdominal Paragangliomas—A Meta-Analysis and Systematic Review of the Literature

Stenman

Zedenius

Juhlin

2019

Cancers

Pheochromocytomas (PCCs) and abdominal paragangliomas (PGLs), collectively abbreviated PPGLs, are neuroendocrine tumors of the adrenal medulla and paraganglia, respectively. These tumors exhibit malignant potential but seldom display evidence of metastatic spread, the latter being the only widely accepted evidence of malignancy. To counter this, pre-defined histological algorithms have been suggested to stratify the risk of malignancy: Pheochromocytoma of the Adrenal Gland Scaled Score (PASS) and the Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP). The PASS algorithm was originally intended for PCCs whereas the GAPP model is proposed for stratification of both PCCs and PGLs. In parallel, advances in terms of coupling overtly malignant PPGLs to the underlying molecular genetics have been made, but there is yet no combined risk stratification model based on histology and the overall mutational profile of the tumor. In this review, we systematically meta-analyzed previously reported cohorts using the PASS and GAPP algorithms and acknowledge a “rule-out” way of approaching these stratification models rather than a classical “rule-in” strategy. Moreover, the current genetic panorama regarding possible molecular adjunct markers for PPGL malignancy is reviewed. A combined histological and genetic approach will be needed to fully elucidate the malignant potential of these tumors.

Case Reports in Endocrinology

“…Giant pheochromocytomas are categorized as tumors >7 cm in size [3,7]. Tese giant pheochromocytomas are reported to be rare with just a few case reports published in literature over the past two decades.…”

Section: Introductionmentioning

confidence: 99%

Giant Pheochromocytoma Diagnosis Confounded by Amphetamine Use

Amin

Gilbert

Barrett

2023

Objective. Diagnosis of giant pheochromocytoma is difficult; patients often lack the classic triad and presence of gross biochemical positivity. At times, presence of sympathetic stimulant drugs can further complicate the clinical picture. Here, we present a case of giant “functional” pheochromocytoma with a history of amphetamine use. Case Description. 37-year-old female presented with a 1-day history of abdominal pain. CT abdomen identified a 12.5 cm heterogeneously enhancing left adrenal mass. Plasma/urine catecholamine and metanephrine levels were markedly elevated with evidence of elevated serum/urine cortisol. However, the patient’s subsequent urine toxicology was found to be positive for amphetamines, which she later admitted to using, 1 week prior to admission. Repeat biochemical workup after 1 week drug washout period showed improvement in both catecholamine and cortisol levels. Given the high degree of suspicion for PCC, an open laparoscopic adrenalectomy was performed with histology confirming SDHB gene mutation positive giant pheochromocytoma. Discussion. Diagnosis of PCC in a patient with a history of amphetamine abuse remains an enigma, to which addition of it being a giant PCC that are rare and typically silent further confounds the clinical picture as seen in this case. Conclusion. PCC could be termed a “chameleon” tumor given its varied clinical presentations and lack of standardized biochemical and radiological data (giant, pheochromocytoma, and amphetamine).